Therapeutic tactics and late results in predominant truncal congenital malformation.

J Cardiovasc Surg (Torino)

Department of Vascular Surgery, University of Athens, Aretaeion Hospital, Greece.

Published: June 2000

Background: Congenital vascular malformations are rare vascular lesions of unknown etiology, non-degenerative or of inflammatory nature, which begin during embryological development; they are characterized by anomalies of the vascular system, apparently due to hemodynamic and metabolic disturbances.

Methods: Our diagnostic and therapeutic management in addition to the late results in 60, mainly truncal cases, out of 265 congenital vascular malformations, are analyzed in the present study. In a 20-year period 25,000 vascular examinations were carried out, among which 265 (1.06%) congenital vascular malformations (CVMs) were discovered, that is 77% (205/265) extra-truncal venous angiomata and 22.7% (60/265) truncal diffuse or localized types. The distribution of the above types was: 22 (36.6%) arteriovenous, 30 (50%) venous and 8 (13.4%) lymphatic.

Results: Surgery was carried out in 48.3% (29/60) of the truncal types of which 37.9% of the cases, on average, recurred 8 years later. Of the 22 arteriovenous malformations 20 patients were operated on (90%), of whom 35% (7/20) had a recurrence; of the 30 venous defects 30% (9/30) were operated on and 44.5% (4/9) of these had a recurrence. The recurrence rate rose to 50% (5/10) in cases of operative therapy of arteriovenous defects and to 20% (2/10) with combined surgical and non surgical methods. The recurrence incidence of venous defects with surgical treatment and sclerotherapy was 54.1% (6/11).

Conclusions: Timely diagnosis, microsurgical techniques and highly specialized surgical and interventional experience are expected to improve these results significantly.

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